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Skornick Y G - - 1983
Only one instance of Dubin-Johnson syndrome associated with cholelithiasis has been reported. We present one additional patient, a 30 year old man with Dubin-Johnson syndrome in whom pancreatitis and cholecystitis, secondary to cholesterol gallstones, developed. A possible cause for the lithogenicity of the bile in the Dubin-Johnson syndrome is considered.
Relevancy Score: Relevancy Score: 1000
Kolarski V - - 1988
Three cases of Dubin-Johnson syndrome in close relatives--brother, sister and a brother's son--are reported. The clinical characteristics, evolution, diagnostic and differential diagnostic problems as well as the treatment and prognosis of the disease are pointed out.
Relevancy Score: Relevancy Score: 943
Nisa Aziz-un - - 2008
A young man presented with recurrent episodes of mild jaundice. Apart from conjugated hyperbilirubinemia, other liver function tests were always normal. Clinical suspicion of Dubin-Johnson syndrome was raised. Liver biopsy showed diffuse deposition of coarse granular dark brown pigment in hepatocytes. Dubin-Johnson syndrome is a benign condition, which results from ...
Relevancy Score: Relevancy Score: 880
Nakata F - - 1979
We described the clinical and biochemical findings in a 32 day-old boy with the Dubin-Johnson syndrome. Only two other patients diagnosed as having the Dubin-Johnson syndrome during neonatal period have been reported in the literature. The ratio of urinary coproporphyrin isomer I of our patient was 97% and that of ...
Relevancy Score: Relevancy Score: 858
Makharia Govind K - - 2002
Dubin-Johnson syndrome is neither complicated by liver cell necrosis nor associated with portal hypertension. We report a 22-year-old man who had recurrent episodes of jaundice (conjugated hyperbilirubinemia) because of Dubin-Johnson syndrome and portal hypertension secondary to portal vein thrombosis. The relationship between Dubin-Johnson syndrome and portal vein thrombosis in this ...
Relevancy Score: Relevancy Score: 839
Shieh C C - - 1990
We report a case of Dubin-Johnson syndrome presenting with neonatal cholestasis. Liver histology was studied during the neonatal period and at 6 years of age. Distinct brownish pigment granules in hepatocytes were noted. This case confirms that Dubin-Johnson syndrome is a cause of neonatal cholestasis.
Relevancy Score: Relevancy Score: 825
Korkmaz Uğur - - 2011
Dubin-Johnson syndrome is a chronic, benign, intermittent jaundice, mostly of conjugated hyperbilirubinemia. The level of bilirubin is not expected to be more than 20 mg/dl in this syndrome. In this article, we report a patient who was evaluated for hyperbilirubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome ...
Relevancy Score: Relevancy Score: 778
Lahmi Farhad F Research Institute for Gastroenterology and Liver Disease, Shahid Beheshti University of Medical sciences Tehran, - - 2011
Elevated serum level of bilirubin is a common manifestation which is occurred in several diseases. Hyperbilirubinemia can manifest either conjugated or unconjugated. Conjugated or direct hyperbilirubinemia usually are caused by hepatocellular diseases or cholestatic liver diseases. Merely conjugated hyperbilirubinemia is the main manifestation of two congenital syndromes, including Dubin-Johnson and ...
Relevancy Score: Relevancy Score: 751
Bar-Meir S - - 1982
99mTc-HIDA cholescintigraphy was performed in 6 patients with Dubin-Johnson syndrome and 1 patient with Rotor syndrome. In the patients with Dubin-Johnson syndrome, the cholescintigrams had a characteristic pattern of delayed visualization or nonvisualization of the gallbladder and bile ducts in the presence of intense, homogeneous, and prolonged visualization of the ...
Relevancy Score: Relevancy Score: 746
Cotton D B - - 1984
A pregnant patient had Dubin-Johnson syndrome. Although the mother had hyperbilirubinemia, there were no untoward fetal effects. The infant's neonatal course, however, was complicated by hepatitis with giant cell transformation.
Relevancy Score: Relevancy Score: 740
Ra Sang Ho - - 2012
Dubin-Johnson syndrome is a rare clinical entity. It shows intermittent symptoms such as chronic or intermittent jaundice, abdominal pain, weakness, nausea, vomiting, anorexia and diarrhea. Symptoms are precipitated or aggravated by pregnancy, alcoholism, surgical procedures and intercurrent disease. Chronic idiopathic jaundice is typical of Dubin-Johnson syndrome and its prognosis is ...
Relevancy Score: Relevancy Score: 724
Nakamura S - - 1983
Massive hepatic resection was carried out on a 42 year-old female who had metastatic lesions of the colonic cancer in right lobe of the liver, which was accompanied by Dubin-Johnson syndrome. The following observations were made. Patient with Dubin-Johnson syndrome tolerated major hepatectomy. As for postoperative regeneration of the liver, ...
Relevancy Score: Relevancy Score: 721
Rubinstein Z J - - 1985
Computed tomography of the liver was performed in 17 patients with Dubin-Johnson syndrome as well as 50 control patients free of liver disease. Multiple readings of liver attenuation values were made in the patients in these two groups and their values compared. The Dubin-Johnson group had liver attenuation values significantly ...
Relevancy Score: Relevancy Score: 716
Viswanathan R - - 1976
Relevancy Score: Relevancy Score: 711
Merdler C - - 1976
11 patients with jaundice of Dubin-Johnson type were treated for 2 weeks with phenobarbital. Serum bilirubin diminished and hepatic clearance of sulfobromophthalein was variably enhanced; other measures of hepatic function were not significantly changed during therapy.
Relevancy Score: Relevancy Score: 709
Enat R - - 1977
Electron microscopy study of a liver biopsy from a patient with the Dubin-Johnson syndrome revealed evidence of severe mitochondrial damage. The changes included membranous whorls, isolated intramitochondrial membranes, myelin figures and large cytoplasmic vacuoles, presumably representing degenerated micochondria. Mitochondrial damage should be looked for in future electron microscopy studies in ...
Relevancy Score: Relevancy Score: 706
Sticova Eva E Center for Experimental Medicine, Institute for Clinical and Experimental Medicine, 14021 Prague 4, Czech Republic. - - 2013
Hyperbilirubinemia has been presumed to prevent the process of atherogenesis and cancerogenesis mainly by decreasing oxidative stress. Dubin-Johnson syndrome is a rare, autosomal recessive, inherited disorder characterized by biphasic, predominantly conjugated hyperbilirubinemia with no progression to end-stage liver disease. The molecular basis in Dubin-Johnson syndrome is absence or deficiency of ...
Relevancy Score: Relevancy Score: 703
Luo Z - - 2000
OBJECTIVE: To investigate the property of pigment granules in the hepatocytes in patients with Dubin-Johnson syndrome. METHOD: Light microscopy, histochemical, immnohistochemical and electron microscopy techniques were used to study the pigment glanules and the expression of S-100 protein and HMB45 in hepatocytes. RESULTS: Histological examination revealed normal lobular architecture and ...
Relevancy Score: Relevancy Score: 700
Mzabi-Regaya Sabah - - 2002
The Dubin-Johnson syndrome (DJS) is an autosomal recessive liver disorder characterized by a chronic conjugated hyperbilirubinemia a dark greenish appearance of liver tissue, a double peaked sulfobromophthalein clearance curve, and a characteristic lysosomal accumulation of black pigment "melanine-like" in the hepatocytes. Laboratory datas indicated an increased urinary excretion of coproporphrin ...
Relevancy Score: Relevancy Score: 698
Coppo M - - 1965
Relevancy Score: Relevancy Score: 688
Kawasaki H - - 1979
Plasma unconjugated bilirubin kinetics were studied by a two-compartment analysis in 10 patients with Dubin-Johnson syndrome (DJS) and 7 normal controls. The clearance of unconjugated bilirubin from plasma was significantly reduced in the patients with DJS as compared to the controls (p less than 0.01). Kinetic analysis of plasma disappearance ...
Relevancy Score: Relevancy Score: 687
Regev Rivka H - - 2002
We report a case of Dubin-Johnson Syndrome in a neonate presenting with severe direct hyperbilirubinemia, which failed to respond to phenobarbital treatment. Ursodeoxycholic Acid added to therapy was well tolerated, and resulted in declining bilirubin concentration. We suggest ursodeoxycholic acid in treatment for Dubin-Johnson Syndrome with severe direct hyperbilirubinemia presenting ...
Relevancy Score: Relevancy Score: 686
Okamura J - - 1980
A 57-year-old man with hepatocellular carcinoma and liver cirrhosis combined with Dubin-Johnson syndrome was successfully treated by extensive right hepatic lobectomy. While the preoperative serum bilirubin level is one of the criteria in determining the indication for radical hepatic resection, it does not play a decisive role in the case ...
Relevancy Score: Relevancy Score: 686
Varma R R - - 1970
A patient is described in whom the Dubin-Johnson syndrome was diagnosed after an attack of acute hepatitis at the age of 21. In the eight years following the hepatitis Dubin-Johnson pigment, initially scanty, developed to classical proportions. The defect in intracellular transport of bilirubin was thought to precede the attack ...
Relevancy Score: Relevancy Score: 681
Otto H U - - 1972
Relevancy Score: Relevancy Score: 672
Fretzayas A - - 2007
Dubin-Johnson syndrome is a worldwide prevalent familial conjugated hyperbilirubinaemia. The identification of multidrug resistance-associated protein 2 provided an understanding of the complex metabolic impairment involved in this syndrome. We report the first family with modified clinical expression of Dubin-Johnson heterozygous state due to genotypic interaction with co-inherited beta and deltabeta ...
Relevancy Score: Relevancy Score: 671
Van De Velde J M - - 1977
Relevancy Score: Relevancy Score: 671
Schillinger H - - 1967
Relevancy Score: Relevancy Score: 671
Sensing H - - 1970
Relevancy Score: Relevancy Score: 671
Ianev P - - 1976
Relevancy Score: Relevancy Score: 669
HALMY L - - 1963
Relevancy Score: Relevancy Score: 669
Park C - - 1990
We compared the pigment of melanosis coli with the pigment of Dubin-Johnson syndrome, melanin, and lipofuscin. The pigment of melanosis coli appeared similar to lipofuscin in that it stained positively with periodic acid-Schiff, oil red-0 and Victoria blue stains and revealed negative reactions to the immunohistochemical stains for S-100 protein ...
Relevancy Score: Relevancy Score: 669
Důra J - - 1973
Relevancy Score: Relevancy Score: 666
Muhammad S - - 1969
Relevancy Score: Relevancy Score: 663
Wolkoff A W - - 1975
Relevancy Score: Relevancy Score: 663
Brandborg L L - - 1972
Relevancy Score: Relevancy Score: 663
Tazuma S - - 1995
Relevancy Score: Relevancy Score: 663
Itoh S - - 2001
Relevancy Score: Relevancy Score: 663
Takino T - - 1977
Relevancy Score: Relevancy Score: 663
MIKULECKY M - - 1960
Relevancy Score: Relevancy Score: 663
Olive D - - 1978
A girl aged 12 with hypogammaglobulinaemia is described who developed Addison's disease with adrenal antibodies at the age of eight. This unusual finding is rarely seen in childhood except in the syndrome of juvenile familial polyendocrinopathy and mucocutaneous moniliasis. By chance the child also had Dubin-Johnson syndrome which could alter ...
Relevancy Score: Relevancy Score: 663
Douglas J G - - 1980
Serum bile salt measurements and intravenous clearance of glycocholate were performed in a woman with Dubin-Johnson syndrome. Fasting conjugated cholate concentration was raised and prolonged intravenous clearance of sodium glycocholate revealed a secondary rise in conjugated cholate concentration after two hours. The intravenous clearance of bromsulphthalein also showed a secondary ...
Relevancy Score: Relevancy Score: 663
Seligsohn U - - 1977
A recent summary of the scanty information about Dubin Johnson Syndrome (DJS) and pregnancy suggests that DJS is accompanied by excessive fetal mortality. This impression could not be corroborated by this study. A fetal mortality of 16% was observed in 75 pregnancies in 18 DJS patients. This value was not ...
Relevancy Score: Relevancy Score: 658
Zhou Lei - - 2013
Dubin-Johnson syndrome (DJS) is unusual during common medical work. Moreover, cholecystolithiasis and choledocholithiasis involvement has not been reported. We describe a case of DJS complicated by cholecystolithiasis and choledocholithiasis. A 49-year-old man accepted by outpatient complained with intermittent cramping pain in right upper abdomen. It is diagnosed as cholecystolithiasis and ...
Relevancy Score: Relevancy Score: 658
Yamashita M - - 1988
Hepatobiliary studies on five patients with Dubin-Johnson syndrome were performed using 99mTc-PMT. Time-activity curves of the liver of the patients showed delayed excretion and that plasma elimination in the patients was prolonged compared to that of normal subjects.
Relevancy Score: Relevancy Score: 654
Arnold C - - 1998
The molecular pathology of hereditary hemochromatosis, Wilson's disease, alpha 1-antitrypsin-deficiency and Dubin-Johnson syndrome could be well characterised during the last years. Diagnosis of hereditary hemochromatosis is reliably confirmed by PCR-augmentation and restriction-analysis. Wilson's disease is a monogenetic disease, which is characterised by over 50 mutations. Molecular diagnosis is complicated by ...
Relevancy Score: Relevancy Score: 654
Pages A - - 1969
Relevancy Score: Relevancy Score: 654
KLATSKY A L - - 1957
Relevancy Score: Relevancy Score: 649
Tyagi S P - - 1994
Relevancy Score: Relevancy Score: 649
Rosenthal P - - 1994
Relevancy Score: Relevancy Score: 649
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